All three of our sons were diagnosed with same rare brain disease after mystery headaches

All three children in an Indiana family have been diagnosed with a rare brain defect that can cause seizures and paralysis. 

The children – aged two, five and 10 – were struck down by seemingly random bouts of dizziness, headaches and sickness that neither doctors nor their parents connected to one another.

Whitney and Ron Niece started to notice something was wrong in June 2022 when their youngest son, Remley, was not as rambunctious as usual and hadn’t been for a wee in over 24 hours.

They took him to the hospital where doctors performed an MRI of the boy’s brain, revealing he had Chari malformation, a one-in-1,000 defect that causes a herniation at the base of the brain which puts pressure on the spinal cord.

Lincoln, Norrin, and Remley Niece were all diagnosed with Chiari Malformation, a rare disorder that causes the brain to extend into the spinal canal

Each of the boys has undergone brain surgery to correct the condition, which only occurs in one in 1,000 people worldwide

Less than a year later, in February 2023, now 10-year-old Lincoln began having headaches and was also diagnosed with Chari malformation.

And just as Remley was about to undergo surgery about six months later, now five-year-old Norrin began to come down with the same headaches suffered by his siblings and he too was diagnosed.

Since their diagnoses, all three boys have had ‘multiple procedures and surgeries’ to correct their condition, which is usually caused by a misshapen or small skull putting pressure on the brain. 

The boys’ father, Ron Niece, told the Greenfield Daily Reporter: ‘It doesn’t get any easier, even after multiple procedures and surgeries.’

According to the Mayo Clinic, there is some evidence suggesting that Chiari malformation can run in families, but research is still early. 

Chiari malformation typically causes issues like headaches, neck pain, poor coordination, dizziness, and trouble swallowing. 

But some people with the condition may never exhibit symptoms or receive a diagnosis, leading doctors to believe the condition is more common.

There are five types of Chiari malformation, with type 1 being the most common and most benign, while type 3 is the rarest and most severe.

This type is associated with debilitating and life-threatening complications.

It’s unclear which type the Niece boys have.  

The Niece siblings all experienced frequent headaches and vomiting, and Remley would often fall over due to his legs randomly giving out.

However, despite his similar symptoms, the Niece’s didn’t initially suspect the condition in Lincoln and attributed the symptoms to his congenital heart condition. It’s unclear which defect he has. 

Lincoln was the first of the siblings to have corrective surgery to prevent his brain from pressing against his spinal cord in March 2023.

But by the time Lincoln went into surgery, Remley had shown signs of delayed development, such as trouble walking and speaking, so he was booked for surgery in August. 

That same month, Norrin received his diagnosis following a period of constant headaches. He went in for surgery in January 2024.  

The boys’ mother, Whitney Niece (pictured here), said that the family is now focused on raising awarness of the disorder, which may cause headaches that are attributed to more common causes

Mrs Niece told the Greenfield Daily Reporter the family has tried to keep a positive attitude in front of the boys, but when Lincoln had his first surgeries, she would sit in the hospital hallway and cry so her sons couldn’t see her.

And while she and her husband have tried to be transparent with their sons about their symptoms and surgeries, they try to make the whole ordeal feel less frightening. 

For Norrin, his parents told him he would go to sleep and wake up with a couple ‘owies.’ 

All three boys underwent a Chiari Malformation Decompression, the standard treatment for the condition. The surgery involves removing a three-centimeter piece of bone toward the back of the skull to relieve pressure. 

However, Norrin and Remley each had complications following the procedure. Both boys developed hydrocephalus, a buildup of fluid in the brain, which required them to have drains placed to remove the excess fluid. 

All three boys finished their surgeries by January 2024 and have been healthy since.

According to Weill Cornell Medicine, nearly all patients who undergo decompression surgery experience partial or total improvement in their symptoms.

Despite restrictions needed to keep the siblings from hitting their heads, such as no bike riding or jumping on the trampoline, the family hopes they can get back to ‘being typical kids.’ It’s unclear how long they will face restrictions. 

Mrs Niece said she is focused on raising awareness of the condition, which she had never heard of until her sons were diagnosed. 

She said: ‘So if we can help one other kid that maybe is struggling with headaches and they’ve been passed off several times, that’s a win in my book.

‘Because a lot of people go in to look for something that is causing a headache and unless you say specifically you’re looking for Chiari, it can get passed off as something else.’